Erythromelalgia

Davis, Mark D.P.; Rooke, Thom W.

doi:10.1007/s11936-006-0008-8

Cited by 31 publications

(25 citation statements)

References 58 publications

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“…While a universally effective treatment regimen for EM has not been established, beta blockers and centrally acting agents (such as anticonvulsants and benzodiazepine) are among the numerous medications that have illustrated clinical efficacy in the management of the signs and symptoms of EM [9–12]. Our patient has had remarkable improvement of her lifelong symptoms on a combination of a beta blocker and a benzodiazepine.…”

Section: Discussionmentioning

confidence: 95%

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Erythema and Burning Pain in the Vulva: A Possible Phenotype of Erythromelalgia

Johnson

Iyer

Eanes³

et al. 2011

Case Reports in Medicine

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Section: Discussionmentioning

confidence: 95%

“…There is no single medication, combination of medications or method of treatment that has been found to be universally helpful in patients suffering from EM [1, 9]. Therefore, providers often rely upon trial and error.…”

Section: Discussionmentioning

confidence: 99%

Erythema and Burning Pain in the Vulva: A Possible Phenotype of Erythromelalgia

Johnson

Iyer

Eanes³

et al. 2011

Case Reports in Medicine

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“…Medications that affect voltage-gated sodium channels show promise, although prostacyclin may provide some benefit and some patients achieved relief with gabapentin or high-dose magnesium [32-34]. Some rare patients with EM may respond well to treatment with carbamazepine especially those with the Na v 1.7channel mutations [35].…”

Section: Discussionmentioning

confidence: 99%

An Egyptian child with erythromelalgia responding to a new line of treatment: a case report and review of the literature

Al-Minshawy

El-Mazary

2014

J Med Case Reports

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IntroductionErythromelalgia is a rare clinical syndrome characterized by episodic erythema, warmth and intense burning pain, which commonly involves the extremities. For those affected, this disorder may lead to significant long-term morbidity. Unfortunately, to date, no definitive therapy is available. This case report describes an Egyptian child with primary erythromelalgia that manifested at an early age and showed partial response to therapy with cetirizine hydrochloride. This anecdotal case report may have a diagnostic value for clinicians who have not seen this disorder.Case presentationA 34-month-old previously healthy right-handed Hamitic boy without any significant past medical history presented at the age of 2 years with episodic bilateral pain in his feet. His mother reported associated warmth and erythema localized to his feet that never extended beyond his ankle joints. This pain is triggered by exertion and/or warm temperature exposure and is relieved by cooling measures. The diagnosis of erythromelalgia was made based on the patient’s medical history and a thorough physical examination during the episodes. No evidence of local or systemic infection was present. Other causes for the symptoms were excluded by a negative extensive diagnostic work-up. Our patient did not respond to ibuprofen (15mg/kg/dose) three times a day but partial improvement with the oral non-sedating antihistaminic cetirizine hydrochloride (2.5mg/kg/once daily) was observed. When the child stopped cetirizine hydrochloride for 1 month as a test, the symptoms became aggravated and were relieved when cetirizine therapy was restarted. Cetirizine hydrochloride had not previously been reported to have this effect in children with erythromelalgia.ConclusionsErythromelalgia is a clinical syndrome of which the etiology, diagnosis and management are controversial. We describe a case of a 34-month-old Egyptian child with primary erythromelalgia that manifested at an early age. We believe that this is the first Egyptian case report of this kind in the literature. Partial response of this patient to cetirizine hydrochloride may grant us a new clue to understanding this mysterious condition.

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“…Various nerve blocks including epidural blocks and sympathetic blockades have been introduced to treat erythromelalgia unresponsive to non-invasive therapies, resulting in considerable efficacy, but no treatment is consistently effective in the management of erythromelalgic patients. For secondary erythromelalgia, treatment of the underlying disorder consists of the most elementary tactics of management [9,10]. …”

Section: Discussionmentioning

confidence: 99%

Secondary Erythromelalgia - A Case Report -

et al. 2013

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Erythromelalgia is a rare neurovascular pain syndrome characterized by a triad of redness, increased temperature, and burning pain primarily in the extremities. Erythromelalgia can present as a primary or secondary form, and secondary erythromelalgia associated with a myeloproliferative disease such as essential thrombocythemia often responds dramatically to aspirin therapy, as in the present case. Herein, we describe a typical case of a 48-year-old woman with secondary erythromelalgia linked to essential thrombocythemia in the unilateral hand. As this case demonstrates, detecting and visualizing the hyperthermal area through infrared thermography of an erythromelalgic patient can assist in diagnosing the patient, assessing the therapeutic results, and understanding the disease course of erythromelalgia.

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Erythromelalgia

Cited by 31 publications

References 58 publications

Erythema and Burning Pain in the Vulva: A Possible Phenotype of Erythromelalgia

Erythema and Burning Pain in the Vulva: A Possible Phenotype of Erythromelalgia

An Egyptian child with erythromelalgia responding to a new line of treatment: a case report and review of the literature

Secondary Erythromelalgia - A Case Report -

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